Angiosarcoma Of The Breast

Cancer is a leading cause of death worldwide and it is likely that you may experience it in your lifetime. Angiosarcoma affects the lining of blood vessels and lymph vessels. It can appear anywhere in the body but one of the most common sites for this type of cancer is the breast. Breast cancer can affect both women and men (more commonly women, with 1 in 8 women diagnosed in their lifetime) and is a serious concern. However, the potential damage that cancer can do is limited if caught early. Therefore, it is important to know how to check if you may be developing cancer and to understand what type of cancer you may have. 

This article will explore the different types of breast cancer and then delve into breast angiosarcoma. This will cover a variety of aspects of the disease including signs and symptoms, diagnosis, treatment and outlook/prognosis. By the end of this article, you should be able to understand this rare form of cancer and how you might be able to detect it yourself. 

Types of breast cancer

We have mentioned angiosarcoma as one type of cancer to affect the breast but there are others. This includes types like inflammatory breast cancer or invasive breast cancer. We will cover a few of the different types in this section but this is not an exhaustive list and does not include all the rarer types. 

Invasive breast cancer is the most common type of breast cancer. This is not a special type of breast cancer but instead refers to tumours that have grown through the lining of the ducts in the breast into the surrounding tissue. This is the most common type with roughly 70% of breast cancer cases being of this type. The second most common type of cancer is invasive lobular breast cancer which accounts for 15% of breast cancer cases. While invasive breast cancer affects the ducts, invasive lobular cancer affects the lobules and grows into the surrounding tissue. Lobules are where the milk is produced in the breast and taken to the nipple via the ducts. Both of these types of cancer are extremely rare in men as they do not possess these tissues.

In addition to invasive breast cancer and invasive lobular breast cancer, there are also non-invasive “mirror” conditions, referred to as ductal breast carcinoma in situ and lobular breast carcinoma in situ. They are similar in that they start in the same areas of tissue, the ducts and the lobules. However, they have not grown and spread into the surrounding tissue. This does not rule out that they can become invasive forms of cancer at a later stage.

Another type of breast cancer is triple-negative breast cancer. This type of cancer refers to cells with no receptors for two hormones, oestrogen and progesterone, and a protein called Her2. Usually, cancer cells have receptors for these substances but these cells do not. Consequently, they cannot be treated with hormone treatment or targeted cancer drugs like trastuzumab, although other drugs such as fam-trastuzumab deruxtecan (Enhertu), paclitaxel (Taxol),  pembrolizumab (Keytruda) or tamoxifen (Soltamox) may be effective. In addition, men can have this type of cancer but breast cancer in men usually has the oestrogen receptor.

A rarer form of cancer is inflammatory breast cancer which affects the lymphatic system. The lymphatic system is part of your immune system and contains various ducts and nodes where your immune cells reside. In addition, this system carries lymph fluid which flows through it and drains away from tissues. In this cancer, the cells block the ducts of this system so that they cannot drain properly, causing a dysfunction of the lymphatic immune system. This causes the tissue to become red and inflamed in the area that is affected. This can cause pain and be confused with an infection due to similar symptoms.

While we have covered some forms of breast cancer, there is a myriad of other, rarer forms related to different tissues in the breast. They can present with different symptoms and require different treatments. One such rare form of cancer is angiosarcoma of the breast which is the main focus of this article.

What is breast angiosarcoma?

Breast angiosarcoma originates in the lining of blood vessels or lymphatic vessels. It makes up less than 1% of breast cancers and is seen in both men and women. Generally, it tends to grow quickly and is difficult to treat. It is so rare that in some cases there is a lack of treatment guidelines, although surgical removal is the usual option. Breast angiosarcoma is divided into three subtypes: primary, secondary and cutaneous. However, cutaneous angiosarcoma is mostly related to the face/head. We will now explore these types.

Types of Angiosarcoma

Primary

Primary angiosarcoma of the breast is the first occurrence of this type of cancer in the breast tissue and may involve the skin of the breast. It is not the result of previous cancer or radiotherapy. This is a rare condition with 17 new cases per 1 million women and it affects younger women in their 30s or 40s.^2,4,5 In addition, due to its rarity and aggressive nature, it has a poor prognosis and diagnosis may be delayed due to its rarity. Once diagnosed, treatment can begin but very little research has been performed to find the optimal therapeutic approach. 

Secondary

Secondary angiosarcoma of the breast is the result of previous cancer in the breast or radiotherapy for previous cancer. It is more common than primary angiosarcoma and occurs in older women (65 to 75 years old).^2,5 It also presents slightly differently from primary angiosarcoma, which it presents with a mass, fullness or swelling in the breast. Secondary angiosarcoma presents with painless bruising and sometimes with a mass but often spread out.  

Cutaneous

Cutaneous angiosarcoma is another type of cancer but this doesn’t affect the breast. This type is more likely to affect the scalp or the face. It is aggressive and can be multi-focal with several areas with cancerous cells. It can be so extensive that surgery to remove the cells is not always successful.³

Signs and symptoms

There are several signs and symptoms of angiosarcoma and it can differ between each subtype and be different for each person. However, the symptoms are generally similar to other types of breast cancer. The signs can include:

• Formation of a lump or thickening in an area of the breast
• A change in the size or shape of the breast
• Dimpling of the skin on the breast
• Nipple which can sink into the breast or a change in shape
• A rash or bruising on the breast or nipple
• A swelling or lump in the armpit

These signs and symptoms can be checked yourself at home. The NHS introduced a 5 point plan to be breast aware to help spot breast cancer. These 5 points are:

• Know what’s normal for you
• Look at your breasts and feel them
• Know what changes to look for (see above)
• Report any changes to a GP without delay
• Attend routine screening if you’re aged 50 to 70

Causes and risk factors

The cause of angiosarcoma is not fully understood especially in the case of primary angiosarcoma. There are some risk factors that have been identified, however. One risk factor is the use of radiotherapy in the past, which is related to secondary angiosarcoma. This risk factor has not been identified as a direct cause, but from case studies in which patients have received radiotherapy in the past.¹ Other risk factors include:

• Chronic lymphoedema¹
• History of radiation (regardless of whether as radiotherapy)¹
• Environmental carcinogens (including vinyl chloride, thorium dioxide and arsenic)¹
• Several genetic syndromes¹

Diagnosis

After you might have spotted signs of breast cancer, you should report to your GP who will refer you to a breast specialist. They may be able to diagnose angiosarcoma from several different tests. These include a mammogram, an ultrasound, a biopsy and an MRI scan. In addition to these techniques, a patient's medical history will be taken into account.^1,2,4,5

A mammogram is an x-ray of the breasts. This is sometimes done through regular screening for breast cancer and can spot cancer up to three years before it can be felt.  If there is any abnormality with a mammogram a specialist will be able to spot it. 

An ultrasound is the same type of test that would be used to assess a pregnancy. It involves It uses high-frequency sound waves that are emitted to the body which then reflect off tissues to a sensor. This will then form an image of the breasts. This will allow a tumour or cancerous cells to be recognised and allow diagnosis. 

Another imaging technique is an MRI, which utilises a large electromagnet. This creates a highly detailed 3D internal image of your body that can be used to diagnose angiosarcoma.

A biopsy is a more invasive technique that can be used to diagnose breast angiosarcoma. This involves taking a section of tissue from an area which is suspected to be the angiosarcoma. This will then be viewed under a microscope to look for features which would suggest that it is a cancerous cell. Due to the invasive nature of this technique, imaging by one of the above techniques would be more likely to be opted for initially.  However, some form of biopsy is almost always required for a firm diagnosis.

Treatments

Due to the rare nature of this type of cancer, there is no established standard treatment or medications that are known to be effective. Normally, removal of the breast (mastectomy) is recommended. If the breast can be saved it will be but only in instances where there is a small lesion. This will remove the cancerous cells from the body and thereby remove the danger.^1,2,4,5

This might then be followed up with chemotherapy or targeted drugs. These drugs reduce the risk of metastasis of the cancer (spreading of the cancer to other tissues). The choice of chemotherapy in angiosarcoma is based on drugs used in the treatment of soft tissue sarcomas.¹ In addition, radiotherapy can be another option. This wouldn’t be used if you have had radiotherapy for a previous cancer.

Outlook/prognosis

Due to the aggressive nature of this type of cancer, there is generally a comparatively poor outlook for the patient. There are several factors that influence an individual’s prognosis. These include tumour size and grade as well as margin status (margin is the surrounding normal tissue around a  tumour that is also removed to confirm the tumour has completely gone). A higher grade and a positive margin (cancerous cells in the normal tissue) are associated with a higher rate of resurgence. There is some doubt regarding size as a factor, with some studies showing a correlation with the outcome and others not. 

In secondary angiosarcoma, one study found that there was a local recurrence rate of 92%.¹ Another study found that there was a recurrence rate of ⅔. These studies also assessed the survival time of patients which ranged from 6 months to 72 months depending on age and whether the cancerous cells were completely removed.^1,2,4,5

Prevention

Angiosarcoma is a rare condition so there is not much research into how the disease can arise. Secondary angiosarcoma is the result of previous cancer or radiotherapy and so cannot be avoided. It is imperative to treat previous cancer than not and the development of secondary angiosarcoma is an unfortunate consequence. However, in the case of primary angiosarcoma, you can avoid the risk factors in an attempt to prevent it.  Radiation can come from medical or occupational exposure and increases the risk of cancer by damaging your DNA. Environmental carcinogens are another risk factor as these chemicals damage our genes leading to cancer. An example of a carcinogen is vinyl chloride. 

Current research and clinical trials

Due to the rarity of breast angiosarcoma, there is little research and current trials into the disease. Most of the studies mentioned above have a relatively small patient size. However, there are currently 83 clinical trials into angiosarcoma which is not specific to the breasts. Mostly, these are investigating new drugs in this condition to improve treatment. Information on these clinical trials can be found here.

Summary

Angiosarcoma of the breast is a rare and aggressive form of cancer that has a poor prognosis. There are three different types including primary, secondary and cutaneous but cutaneous is more likely to affect the head and scalp. There are signs and symptoms that can indicate breast cancer and you can check yourself for lumps which may indicate cancer. This would lead to a diagnosis which can happen from several imaging techniques or from a biopsy. As this type of cancer is rare, there is no standard treatment but it is generally recommended that the breast be removed and then followed by chemotherapy. Overall, there is a poor prognosis but by checking your breasts and regular screening, you would be able to catch it early for better survival.